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https://www.readbyqxmd.com/read/29454680/cardiomyopathy-in-the-pediatric-patients
#1
REVIEW
Shi-Min Yuan
Pediatric cardiomyopathies are a group of myocardial diseases with complex taxonomies. Cardiomyopathy can occur in children at any age, and it is a common cause of heart failure and heart transplantation in children. The incidence of pediatric cardiomyopathy is increasing with time. They may be associated with variable comorbidities, which are most often arrhythmia, heart failure, and sudden death. Medical imaging technologies, including echocardiography, cardiac magnetic resonance, and nuclear cardiology, are helpful in reaching a diagnosis of cardiomyopathy...
January 31, 2018: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29440775/genetic-defects-in-mtdna-encoded-protein-translation-cause-pediatric-mitochondrial-cardiomyopathy-with-early-onset-brain-disease
#2
Rick Kamps, Radek Szklarczyk, Tom E Theunissen, Debby M E I Hellebrekers, Suzanne C E H Sallevelt, Iris B Boesten, Bart de Koning, Bianca J van den Bosch, Gajja S Salomons, Marisa Simas-Mendes, Rob Verdijk, Kees Schoonderwoerd, Irenaeus F M de Coo, Jo M Vanoevelen, Hubert J M Smeets
This study aims to identify gene defects in pediatric cardiomyopathy and early-onset brain disease with oxidative phosphorylation (OXPHOS) deficiencies. We applied whole-exome sequencing in three patients with pediatric cardiomyopathy and early-onset brain disease with OXPHOS deficiencies. The brain pathology was studied by MRI analysis. In consanguineous patient 1, we identified a homozygous intronic variant (c.850-3A > G) in the QRSL1 gene, which was predicted to cause abnormal splicing. The variant segregated with the disease and affected the protein function, which was confirmed by complementation studies, restoring OXPHOS function only with wild-type QRSL1...
February 13, 2018: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/29439302/pediatric-viral-myocarditis-a-review
#3
Teresa Frey, Nofil Arain
Pediatric myocarditis is a common pediatric illness most commonly secondary to a preceding viral infection. It is a leading cause of acquired heart failure, cardiomyopathy, and cardiac transplantation in pediatrics. Due to the variability in presentation, the diagnosis is often unrecognized until later in the disease course. It should be considered in the differential diagnosis of all children presenting with respiratory distress, since this is the most common presentation. Imagining modalities, such as cardiac magnetic resonance imaging have become a useful diagnostic tool in recent years; however, endomyocardial biopsy remains the gold standard diagnostic test...
January 2018: South Dakota Medicine: the Journal of the South Dakota State Medical Association
https://www.readbyqxmd.com/read/29431102/pediatric-dilated-cardiomyopathy-associated-lrrc10-leucine-rich-repeat-containing-10-variant-reveals-lrrc10-as-an-auxiliary-subunit-of-cardiac-l-type-ca2-channels
#4
Marites T Woon, Pamela A Long, Louise Reilly, Jared M Evans, Alexis M Keefe, Martin R Lea, Carl J Beglinger, Ravi C Balijepalli, Youngsook Lee, Timothy M Olson, Timothy J Kamp
BACKGROUND: Genetic causes of dilated cardiomyopathy (DCM) are incompletely understood. LRRC10 (leucine-rich repeat-containing 10) is a cardiac-specific protein of unknown function. Heterozygous mutations in LRRC10 have been suggested to cause DCM, and deletion of Lrrc10 in mice results in DCM. METHODS AND RESULTS: Whole-exome sequencing was carried out on a patient who presented at 6 weeks of age with DCM and her unaffected parents, filtering for rare, deleterious, recessive, and de novo variants...
February 3, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29428438/no-obesity-paradox-in-pediatric-patients-with-dilated-cardiomyopathy
#5
Chesney D Castleberry, John L Jefferies, Ling Shi, James D Wilkinson, Jeffrey A Towbin, Ryan W Harrison, Joseph W Rossano, Elfriede Pahl, Teresa M Lee, Linda J Addonizio, Melanie D Everitt, Justin Godown, Joseph Mahgerefteh, Paolo Rusconi, Charles E Canter, Steven D Colan, Paul F Kantor, Hiedy Razoky, Steven E Lipshultz, Tracie L Miller
OBJECTIVES: This study aimed to examine the role of nutrition in pediatric dilated cardiomyopathy (DCM). BACKGROUND: In adults with DCM, malnutrition is associated with mortality, whereas obesity is associated with survival. METHODS: The National Heart, Lung, and Blood Institute-funded Pediatric Cardiomyopathy Registry was used to identify patients with DCM and categorized by anthropometric measurements: malnourished (MN) (body mass index [BMI] <5% for ≥2 years or weight-for-length <5% for <2 years), obesity (BMI >95% for age ≥2 years or weight-for-length >95% for <2 years), or normal bodyweight (NB)...
February 1, 2018: JACC. Heart Failure
https://www.readbyqxmd.com/read/29428437/obesity-and-prognosis-in-pediatric-dilated-cardiomyopathy-no-paradox-or-adverse-effects
#6
EDITORIAL
Carl J Lavie, Hector O Ventura
No abstract text is available yet for this article.
February 1, 2018: JACC. Heart Failure
https://www.readbyqxmd.com/read/29425530/ventricular-assist-devices-for-neonates-and-infants
#7
REVIEW
Katsuhide Maeda, David N Rosenthal, Olaf Reinhartz
Heart transplant waitlist survival in pediatric patients has been substantially improved since the introduction of pediatric-specific ventricular assist device. In neonates and infants, however, the waitlist mortality remains very high. The only long-term device currently approved for use in the United States is the Berlin Heart EXCOR, but this device has several important limitations because of the paracorporeal, pulsatile nature of the underlying technology. We reviewed Stanford ventricular assist experience on patients less than 1 year old since 2004...
March 2018: Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual
https://www.readbyqxmd.com/read/29395891/hemodynamic-support-of-a-15-year-old-waiting-for-a-heart-transplant-is-there-a-role-for-levosimendan-in-pediatric-heart-failure
#8
I Goyer, D Brossier, B Toledano
Decompensated heart failure in children requires rapid and aggressive support. In refractory cases, invasive supportive care is essential to ensure cardiac output. This results in lengthy pediatric intensive care unit (PICU) stays, secondary morbidity, and high cost. Levosimendan may help palliate the pitfalls encountered with the usual treatment. It has been shown to improve hemodynamics and decrease morbidity and mortality from heart failure in adult trials and pediatric cohorts. We report the case of a 15-year-old boy with dilated cardiomyopathy and refractory ventricular dysfunction who was weaned from continuous inotropes and discharged from the PICU with levosimendan while waiting for heart transplantation...
January 26, 2018: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29367541/diagnostic-yield-of-whole-exome-sequencing-in-pediatric-dilated-cardiomyopathy
#9
Pamela A Long, Jared M Evans, Timothy M Olson
Dilated cardiomyopathy (DCM) is a heritable, genetically heterogeneous disorder characterized by progressive heart failure. DCM typically remains clinically silent until adulthood, yet symptomatic disease can develop in childhood. We sought to identify the genetic basis of pediatric DCM in 15 sporadic and three affected-siblings cases, comprised of 21 affected children (mean age, five years) whose parents had normal echocardiograms (mean age, 39 years). Twelve underwent cardiac transplantation and five died with severe heart failure...
August 8, 2017: Journal of Cardiovascular Development and Disease
https://www.readbyqxmd.com/read/29362845/hypertrophic-cardiomyopathy-genotype-prediction-models-in-a-pediatric-population
#10
Randa Newman, John Lynn Jefferies, Clifford Chin, Hua He, Amy Shikany, Erin M Miller, Ashley Parrott
The Toronto Hypertrophic Cardiomyopathy (HCM) Genotype Score and Mayo HCM Genotype Predictor are risk assessment models developed to estimate a patient's likelihood of testing positive for a pathogenic variant causative of HCM. These models were developed from adult populations with HCM based on factors that have been associated with a positive genotype and have not been validated in external populations. The purpose of this study was to evaluate the overall predictive abilities of these models in a clinical pediatric HCM setting...
January 24, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29343586/a-case-of-cardiomyopathy-due-to-premature-ductus-arteriosus-closure-the-flip-side-of-paracetamol
#11
Ingrid Anne Mandy Schierz, Mario Giuffrè, Ettore Piro, Simona La Placa, Giovanni Corsello
Paracetamol (acetaminophen or N-acetyl-p-aminophenol) is considered a safe analgesic and antipyretic nonsteroidal antiinflammatory drug commonly used during pediatric ages and during pregnancy. We report on a term neonate with closed ductus arteriosus, severe cardiomyopathy, right ventricular dysfunction, and functional stenosis of pulmonary arteries at birth after maternal self-medication with paracetamol and consumption of polyphenol-rich foods in late pregnancy. This drug, especially when associated with other vasoconstrictors (such as polyphenols), interferes with prostaglandin metabolism, which seriously accentuates the intrauterine ductus arteriosus constriction and leads to pharmacologic adverse events...
February 2018: Pediatrics
https://www.readbyqxmd.com/read/29332781/pediatric-extracorporeal-cardiopulmonary-resuscitation-patient-with-traumatic-subarachnoid-hemorrhage-and-takotsubo-syndrome
#12
Thomas E Pearson, Meg A Frizzola, Marc A Priest, Monica F Rochman, Curtis D Froehlich
Takotsubo syndrome is rare in pediatric patients but must be considered in patients with subarachnoid hemorrhage with pulmonary edema and cardiomyopathy. A systematic, collaborative approach is needed to facilitate emergent transfer of patients where extracorporeal cardiopulmonary resuscitation (e-CPR) is used as a lifesaving measure. Extracorporeal membrane oxygenation (ECMO) use in transport requires preplanning, role delineation, resources, and research efforts to be successful. We present an unusual transport case of successful e-CPR/ECMO treatment of Takotsubo syndrome in a 12-year-old boy with an isolated traumatic intracranial injury, cardiomyopathy with pulmonary edema, and multiple cardiac arrests...
January 2018: Air Medical Journal
https://www.readbyqxmd.com/read/29260263/systematic-literature-review-on-the-incidence-and-prevalence-of-heart-failure-in-children-and-adolescents
#13
REVIEW
Robert E Shaddy, Aneesh Thomas George, Thomas Jaecklin, Eimear Nic Lochlainn, Lalit Thakur, Rumjhum Agrawal, Susan Solar-Yohay, Fabian Chen, Joseph W Rossano, Thomas Severin, Michael Burch
While the epidemiology of adult heart failure has been extensively researched, this systematic review addresses the less well characterized incidence and prevalence of pediatric HF. The search strategy used Cochrane methodology and identified 83 unique studies for inclusion. Studies were categorized according to whether the HF diagnosis was reported as primary (n = 10); associated with other cardiovascular diseases (CVDs) (n = 49); or associated with non-CVDs (n = 24). A narrative synthesis of the evidence is presented...
December 20, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29248563/exercise-participation-and-shared-decision-making-in-patients-with-inherited-channelopathies-and-cardiomyopathies
#14
Susan P Etheridge, Elizabeth V Saarel, Matthew W Martinez
Sports eligibility and disqualification for patients with cardiac diseases are important considerations for adult and pediatric cardiologists. The 2005 guidelines that addressed this have recently been revised and updated and the new guidelines advocate for a shared decision making approach where the well-informed athlete and family participate in the discussion. In this review, we focus on the benefits of sports participation and review the revised guidelines related to sports participation in patients with channelopathies and cardiomyopathies...
December 14, 2017: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://www.readbyqxmd.com/read/29238322/acute-ischemic-pediatric-stroke-management-an-extended-window-for-mechanical-thrombectomy
#15
Ashish Kulhari, Elizabeth Dorn, Jonathan Pace, Vilakshan Alambyan, Stephanie Chen, Osmond C Wu, Macym Rizvi, Anthony Hammond, Ciro Ramos-Estebanez
Ischemic stroke is a rare condition to afflict the pediatric population. Congenital cardiomyopathy represents one of several possible etiologies in children. We report a 9-year-old boy who developed right middle cerebral artery stroke secondary to primary restrictive cardiomyopathy. In the absence of pediatric guidelines, the child met adult criteria for mechanical thrombectomy given the small core infarct and large penumbra. The literature suggests children may benefit from mechanical thrombectomy in carefully selected cases...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/29230826/evolution-of-biventricular-loading-condition-in-pediatric-lvad-patient-a-prospective-and-observational-study
#16
Arianna Di Molfetta, Roberta Iacobelli, Sergio Filippelli, Giorgia Grutter, Gianluigi Perri, Francesca Iodice, Luciano Pasquini, Paolo Guccione, Antonio Amodeo
The aim of this study was to describe the echocardiographic trend of left ventricular (LV) and right ventricular (RV) function after implantation of a pulsatile flow left ventricular assist device (LVAD) in children. From 2013 to 2016, we prospectively evaluated 13 consecutive pediatric Berlin Heart EXCOR LVAD patients. Clinical and echocardiographic data were collected at baseline, within 24 h after implantation and monthly until LVAD explant. Median age and weight at the implantation was 8 (4-23) months and 5 (4...
December 12, 2017: Artificial Organs
https://www.readbyqxmd.com/read/29212898/genetic-testing-in-pediatric-left-ventricular-noncompaction
#17
Erin M Miller, Robert B Hinton, Richard Czosek, Angela Lorts, Ashley Parrott, Amy R Shikany, Richard F Ittenbach, Stephanie M Ware
BACKGROUND: Left ventricular noncompaction (LVNC) can occur in isolation or can co-occur with a cardiomyopathy phenotype or cardiovascular malformation. The yield of cardiomyopathy gene panel testing in infants, children, and adolescents with a diagnosis of LVNC is unknown. By characterizing a pediatric population with LVNC, we sought to determine the yield of cardiomyopathy gene panel testing, distinguish the yield of testing for LVNC with or without co-occurring cardiac findings, and define additional factors influencing genetic testing yield...
December 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/29206935/report-of-a-myocarditis-outbreak-among-pediatric-patients-human-herpesvirus-7-as-a-causative-agent
#18
Rahmi Ozdemir, Mehmet Kucuk, Saime Ergen Dibeklioglu
Background: The etiology of myocarditis in children has not yet been completely elucidated. Objective: Medical records of eight pediatric patients diagnosed with acute myocarditis within a 41-day period in a small-town hospital were retrospectively analyzed. Methods: We examined antibody titers of adenovirus, Epstein-Barr virus, herpes simplex virus, respiratory syncytial virus, varicella-zoster virus and cytomegalovirus in peripheral blood...
November 30, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/29188317/genetic-testing-in-pediatric-cardiomyopathy
#19
Chalani D Ellepola, Linda M Knight, Peter Fischbach, Shriprasad R Deshpande
Genetic testing is recommended in patients with dilated cardiomyopathy (DCM); however, limited studies demonstrate high yields of genetic testing in non-hypertrophic (HCM) patients. Furthermore, there is sparse genotype-phenotype data in pediatric DCM patients. We performed a retrospective review of 70 consecutive probands with cardiomyopathy (non-HCM) who underwent genetic evaluation. Mean age at presentation was 5.48 years. Echocardiography revealed mean ejection fraction of 32.4%. The LVEDd z score ranged from - 5...
November 29, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/29180164/th-17-cytokines-are-associated-with-severity-of-trypanosoma-cruzi-chronic-infection-in-pediatric-patients-from-endemic-areas-of-mexico
#20
Mariana De Alba-Alvarado, Paz María Salazar-Schettino, Luis Jiménez-Álvarez, Margarita Cabrera-Bravo, Cecilia García-Sancho, Edgar Zenteno, Clara Vazquez-Antona, Alfredo Cruz-Lagunas, Joaquín Zúñiga, Martha Irene Bucio-Torres
In Chagas disease the clinical, acute and chronic manifestations are the result of the interaction between the parasite and the host factors. The balance between inflammatory and anti-inflammatory immune responses is essential for the increase or resolution of the manifestations in individuals infected with T. cruzi. To identify if children with chronic Chagas disease and heart injury is related with non-regulated Th1, Th2 and Th17 responses. We included 31 children with T. cruzi confirmed chronic infection from endemic areas of Mexico...
November 24, 2017: Acta Tropica
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